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List of works by Hibiki Kawamata

A kinetic assay of mitochondrial ADP-ATP exchange rate in permeabilized cells.

scientific article

ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response

scientific article published on 14 March 2019

Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis

scientific article published on February 2014

Accelerated transsulfuration metabolically defines a discrete subclass of amyotrophic lateral sclerosis patients

scientific article published on 31 July 2020

Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells

scientific article

Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients

scientific article

Correction: Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases

scientific article published on 21 December 2017

Distal denervation in the SOD1 knockout mouse correlates with loss of mitochondria at the motor nerve terminal

scientific article published on 10 May 2019

Experimental strategy to identify genes susceptible to oxidative stress in nigral dopaminergic neurons

scientific article published on June 2004

Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients.

scientific article published on 24 October 2017

Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria

scientific article published on 20 August 2008

Measurement of ADP-ATP exchange in relation to mitochondrial transmembrane potential and oxygen consumption

scientific article

Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis

scientific article published on 14 August 2015

Mitochondrial dysfunction and intracellular calcium dysregulation in ALS

scientific article

Modulation of the IGF1R-MTOR pathway attenuates motor neuron toxicity of human ALS SOD1<sup>G93A</sup> astrocytes

scientific article published on 22 March 2021

Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy ⬇️

scientific article published on 01 March 2022

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo

scientific article

Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice.

scientific article published on 21 September 2003

Neurotoxicity and behavioral deficits associated with Septin 5 accumulation in dopaminergic neurons

scientific article published on August 2005

Nrf2 signaling links ER oxidative protein folding and calcium homeostasis in health and disease

scientific article published on 28 October 2019

Parkin is a disease modifier in the mutant SOD1 mouse model of ALS

scientific article published on 01 October 2018

Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases

scientific article published on 22 November 2017

The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation

scientific article published on 8 March 2013

adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria

scientific article published on 17 May 2011

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